Dermatomyositis treatment

Juvenile dermatomyositis (JDM) is perhaps the most uniform, in terms of clinical and histopathological features, of the whole PM/DM disease complex. Presentation may be before 5 years of age with peak incidence between 8 and 12 years. The disease may remit and recur until well into young adult life. The skin lesions include a facial rash in butterfly distribution across nose and cheeks. Erythematous skin changes are seen over extensor surfaces of joints, especially knees, knuckles and elbows. Muscle involvement is generally evident some time later and takes the form of weakness and stiffness, particularly affecting shoulder and pelvic musculature. Proximal muscles are often worse affected than distal muscles and extensors worse than flexors. In the absence of prompt and effective treatment contractures may occur at elbows, ankles, knees, and hips. Subcutaneous calcification and skin ulceration may be found calcification of deeper-lying connective tissue may be apparent on X-ray. 

Although the most common methotrexate dosing regimens for the treatment of rheumatoid arthritis are 15 or 17.5 mg weekly, there is an increased effect up to 30 or 35 mg weekly. The drug decreases the rate of appearance of new erosions. Evidence supports its use in juvenile chronic arthritis, and it has been used in psoriasis, psoriatic arthritis, polymyositis, dermatomyositis, Wegener s granulomatosis, giant cell arteritis, subacute lupus erythematosus, and vasculitis. 

Dietrich LL, Bridges AJ, Albertini MR. Dermatomyositis after interferon alpha treatment. Med Oncol 2000 17(l) 64-9. 

Choy EHS, Isenberg DA. Treatment of dermatomyositis and polymyositis. Rheumatology 2002 41 7-13. 

Treatments with recombinant therapeutic cytokines occasionally induce autoimmune phenomena. In particular, IFN-a may cause or exacerbate autoimmune effects, in particular thyroiditis, but also autoimmune thrombocytopenia or autoimmune hepatitis, and systemic lupus erythematosus has been reported. Relationships between IFN-a treatment and dermatomyositis (Dietrich et al., 2000) and diabetes mellitus (Fabris et al., 2003) have been observed. In addition, increased levels of various autoantibodies have been found in patients receiving IFN-a (Vial et al., 2002). Monzani et al. 

D-penicillamine is so named because it was first isolated as an amine, from the degradation products of penicillin by Abraham et al [87]. Later studies showed the characteristic chemical behavior of D-penicillamine which involve three types of reactions, formation of disulphide links, formation of thiazolidine rings, and formation of metal complexes and chelates [67]. It was first used in 1956 in the treatment of Wilson s disease [88]. D-penicillamine has since been used in the treatment of many diseases, such as cystinuria [89], rheumatoid arthritis [90-92], systemic sclerosis [93], primary bdiary cirrhosis [94], heavy metal poisoning due to lead [95], cadmium [%], and mercury [97], and hyperviscosity syndrome [99]. In rheumatoid arthritis, D-peni-cdlamine has been widely accepted as an effective second line treatment. Despite of its effectiveness, it causes many adverse effects, such as skin rashes [99,100], taste abnormalities [100,101], hepatic dysfunction [102-104], gastrointestinal toxiciiy [99,105], proteinuria [100,106], hematuria [107, 108], thrombocytopenia [92, 109], aplastic anemia [110], lupus-like syndrome [111, 112], Goodpasture s-tike pulmonary renal syndrome [113-115], vasculitis [116,117], myasthenia gravis [118-122], polymyositis [123, 124], and dermatomyositis [125]. 

THERAPEUTIC USES FDA-approved dermatological uses of hydroxychloroquine include treatment of discoid and systemic lupus erythematosus. Unapproved but first-line uses include treatment of dermatomyositis, porphyria cutanea tarda, polymorphous light eruption, sarcoidosis, eosinophilic fasciitis, lymphocytic infiltrate of Jessner, lymphocytoma cutis, solar urticaria, granuloma annulare, and some forms of panniculitis. 

Lundgren R, Back O, Wiman LG (1975) Pulmonary lesions and autoimmune reactions after long-term nitrofurantoin treatment. Scand Respir Dis 56 208 Lynn KL, Little PJ (1976) Reaction to trimoxazole. NZ Med J 377 78 Mackie BS, Mackie LE (1979) Systemic lupus erythematosus-dermatomyositis induced by sulphacetamide eye drops. Aust J Dermatol 20 49 Maddrey W, Boitnott J (1977) Drug-induced chronic liver disease. Gastroenterology 72 1348... 

Some diseases, including dermatosclerosis, dermatomyositis, systemic lupus ery-thematosis, psoriasis, and myasthenia gravis, etc. may be the result of a disorder of immune responses. The Ganoderma lucidum spore extract showed inhibiting effects on DHT, decreased the primary antibody response in mice and also, in a dose related manner, suppression of murine splenocytes or human tonsil mononuclear cell proliferation in vitro. It inhibited a mixed lymphocyte reaction and decreased the IL-2 level. These results indicate that the inhibitory actions on both the cellular and humoral immunity might explain the use of this drug for the treatment of autoimmune disorders [52]. 

Manlhiot C, Tyrrell PN, Liang L, Atkinson AR, Lau W, Feldman BM. Safety of intravenous immunoglobulin in the treatment of juvenile dermatomyositis adverse reactions are associated with immunoglobulin A content. Pediatrics 2008 121(3) e626-30. 

Dermatomyositis occurred in a 57-year-old patient who took interferon beta for multiple sclerosis [56 ]. Immunohistochemical staining of skin biopsies for myxovirus-resistance protein A (a surrogate marker of cutaneous type I interferon signalling) showed increased staining that correlated temporally with interferon beta treatment and subsequent disease activity. In vitro treatment with interferon beta of peripheral blood mononuclear cells isolated from this patient showed enhanced type I interferon signaling, assessed by interferon-induced gene expression profiles. 

Schnabel A, Reuter M, Biederer J, et al. Interstitial lung disease in polymyositis and dermatomyositis clinical course and response to treatment. Semin Arthritis Rheum 2003 32 273-284. 

Takada K, Kishi J, Miyasaka N. Step-up versus primary intensive approach to the treatment of interstitial pneumonia associated with dermatomyositis/polymyositis a retrospective study. Mod Rheumatol 2007 17 123-130. 

Engel WK, BorensteinA, DeVivo DC etal. (1972) High-single-dose alternate-day prednisone (HSDAD-PRED) in the treatment of dermatomyositis/polymyositis complex. Trans Am Neurol Assn 97, 272-275. 

Dermatomyositis causes a moderately or rapidly progressive muscle weakness, with or without skin manifestations. Muscle biopsy is nearly always diagnostic. Treatment is typically begun with a glucocorticoid, usually prednisone, and it is often effective in restoring good to excellent strength. 

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